Intellectual Functions and Personality in Subjects with Noncongenital Myotonic Muscular Dystrophy

Abstract

Mental retardation and personality disorders are commonly described among the symptoms of myotonic dystrophy. Nevertheless, this tendency is not supported by systematic studies performed on large samples, whose results are controversial. We studied the cognitive functions and personalities of a group of 28 patients, in whom myotonic dystrophy had commenced in juvenile or adult life. The severity of the disease was variable, but all subjects were self-sufficient. Only 7.1% of subjects showed low intelligence with deterioration of perceptual-motor functions. This was not correlated with the severity of their disease. Women had a substantially lower mean Wechsler-Bellevue score than men. The personality function study of the entire group showed no change of psychiatric relevance but did present a depressive attitude with marked somatic concern and difficulties in establishing relationships in social life.